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Search on : Reticulocytosis [Words]
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[PMID]: 29506817
[Au] Autor:Pugi J; Carcao M; Drury LJ; Langer JC
[Ad] Address:Division of Hematology/Oncology, Child Health Evaluative Sciences, Research Institute, Hospital for Sick Children and Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
[Ti] Title:Results after laparoscopic partial splenectomy for children with hereditary spherocytosis: Are outcomes influenced by genetic mutation?
[So] Source:J Pediatr Surg;, 2018 Feb 08.
[Is] ISSN:1531-5037
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Laparoscopic partial splenectomy (LPS) theoretically maintains long-term splenic immune function for children with hereditary spherocytosis (HS). Our goal was to review our results after LPS and to determine if specific genetic mutations influence outcome. METHODS: All children with HS undergoing LPS between 2005 and 2016 were reviewed. RESULTS: Thirty-one children underwent LPS (16 male) at a median age of 9 (range 2-18) years. All experienced an increase in hemoglobin and decrease in reticulocyte count early after LPS and at last follow-up. Twenty-two were sent for genetic analysis. Mutations in α-spectrin, ß-spectrin, and Ankyrin were identified in 6, 5, and 11 patients, respectively. Gene mutation was not correlated with complications, perioperative transfusion, length of hospital stay, or median hemoglobin, platelet, or reticulocyte counts. Three children required completion splenectomy at 10.9, 6.9, and 3.2years post-LPS, each with a different gene mutation. CONCLUSIONS: LPS is effective in reversing anemia and reducing reticulocytosis. So far less than 10% have required completion splenectomy, and those children did benefit from delaying the risks of asplenia. In this preliminary analysis, genetic mutation did not influence outcome after LPS. A larger multicenter study is necessary to further investigate potential correlations with specific genetic mutations. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: IV.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180306
[Lr] Last revision date:180306
[St] Status:Publisher

  2 / 885 MEDLINE  
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[PMID]: 29152309
[Au] Autor:Song SH; Kang SK; Choi WJ; Kwak KM; Lee DH; Kang DY; Lee SH
[Ad] Address:Department of Occupational and Environmental Medicine, Gachon University Gil Medical Center, 21, Namdongdaero774 beon-gil, Nadong-gu, Incheon, 21565 Republic of Korea.
[Ti] Title:Reticulocytosis in screen-printing workers exposed to 2-butoxyethanol and 2-ethoxyethanol.
[So] Source:Ann Occup Environ Med;29:54, 2017.
[Is] ISSN:2052-4374
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Background: Studies on the hematologic toxicity of ethylene glycol ethers in humans are limited. Therefore, the aim of this study was to examine the association between exposure to solvents (containing 2-butoxyethanol and 2-ethoxyethanol) and hematological effects. Methods: Thirty-four screen-printing workers who were exposed to 2-butoxyethanol and 2-ethoxyethanol and 37 non-exposed clerical workers were selected using data from the health care facilities that provided regular health screening services. Student's t-tests and Pearson's chi-square tests were used to compare differences in hematological parameters between the exposed and the control groups. A multivariate analysis was performed using the multiple logistic regression models to adjust for other variables. Results: The chi-square test showed the reticulocyte percentages and corrected reticulocyte counts to be significantly higher in the exposed group. The t-tests showed a significant increase in white blood cell counts, reticulocyte percentages, and corrected reticulocyte count (i.e., reticulocyte index) in the exposed group, with -values of 0.002, 0.004, and 0.002, respectively. Multivariate analysis showed the odds ratio for the corrected reticulocyte counts to be 16.30 for the exposed group, when compared with that of the control group. Conclusions: Exposure to 2-butoxyethanol and 2-ethoxyethanol was significantly associated with reticulocytosis, necessitating the implementation of preventive measures for workers prone to occupational exposure to ethylene glycol ethers.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 171122
[Lr] Last revision date:171122
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.1186/s40557-017-0210-z

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[PMID]: 29117957
[Au] Autor:Dlamini N; Saunders DE; Bynevelt M; Trompeter S; Cox TC; Bucks RS; Kirkham FJ
[Ad] Address:From Developmental Neurosciences (N.D., F.J.K.), UCL Great Ormond Street Institute of Child Health, London, UK; Hospital for Sick Children (N.D.), Toronto, Canada; Department of Radiology (D.E.S., M.B., T.C.C.), Great Ormond Street Hospital for Children NHS Trust, London, UK; University of Western A
[Ti] Title:Nocturnal oxyhemoglobin desaturation and arteriopathy in a pediatric sickle cell disease cohort.
[So] Source:Neurology;, 2017 Nov 08.
[Is] ISSN:1526-632X
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:OBJECTIVE: The purpose of this study of sickle cell disease (SCD) was to determine whether arteriopathy, measurable as intracranial vessel signal loss on magnetic resonance angiography (MRA), was associated with low nocturnal hemoglobin oxygen saturation (SpO ) or hemolytic rate, measurable as reticulocytosis or unconjugated hyperbilirubinemia. METHODS: Ninety-five East London children with SCD without prior stroke had overnight pulse oximetry, of whom 47 (26 boys, 39 hemoglobin SS; mean age 9.1 ± 3.1 years) also had MRA, transcranial Doppler (TCD), steady-state hemoglobin, and reticulocytes within 34 months. Two radiologists blinded to the other data graded arteriopathy on MRA as 0 (none) or as increasing severity grades 1, 2, or 3. RESULTS: Grades 2 or 3 arteriopathy (n = 24; 2 with abnormal TCD) predicted stroke/TIA compared with grades 0 and 1 (log-rank χ [1, n = 47] = 8.1, = 0.004). Mean overnight SpO correlated negatively with reticulocyte percentage ( = -0.387; = 0.007). Despite no significant differences across the degrees of arteriopathy in genotype, mean overnight SpO was higher ( < 0.01) in those with grade 0 (97.0% ± 1.6%) than those with grades 2 (93.9 ± 3.7%) or 3 (93.5% ± 3.0%) arteriopathy. Unconjugated bilirubin was not associated but reticulocyte percentage was lower ( < 0.001) in those with grade 0 than those with grades 2 and 3 arteriopathy. In multivariable logistic regression, lower mean overnight SpO (odds ratio 0.50, 95% confidence interval 0.26-0.96; < 0.01) predicted arteriopathy independent of reticulocyte percentage (odds ratio 1.47, 95% confidence interval 1.15-1.87; = 0.003). CONCLUSION: Low nocturnal SpO and reticulocytosis are associated with intracranial arteriopathy in children with SCD. Preventative strategies might reduce stroke risk.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 171109
[Lr] Last revision date:171109
[St] Status:Publisher

  4 / 885 MEDLINE  
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[PMID]: 29109103
[Au] Autor:von Wnuck Lipinski K; Weske S; Keul P; Peters S; Baba HA; Heusch G; Gräler MH; Levkau B
[Ad] Address:Institute for Pathophysiology, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany.
[Ti] Title:Hepatocyte nuclear factor 1A deficiency causes hemolytic anemia in mice by altering erythrocyte sphingolipid homeostasis.
[So] Source:Blood;, 2017 Nov 06.
[Is] ISSN:1528-0020
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The Hepatocyte Nuclear Factor (HNF) family regulates complex networks of metabolism and organ development. Human mutations in its prototypical member HNF1A cause maturity-onset of diabetes of the young (MODY) type 3. In this study, we identified an important role for HNF1A in the preservation of erythrocyte membrane integrity, calcium homeostasis and osmotic resistance through a yet unrecognized link of HNF1A to sphingolipid homeostasis. HNF1A mice displayed microcytic hypochromic anemia with reticulocytosis that was partially compensated by avid extramedullary erythropoiesis at all erythroid stages in the spleen thereby excluding erythroid differentiation defects. Morphologically, HNF1A erythrocytes resembled acanthocytes and displayed increased phosphatidylserine exposure, high intracellular calcium, and elevated osmotic fragility. Sphingolipidome analysis by mass spectrometry revealed substantial and tissue-specific sphingolipid disturbances in several tissues including erythrocytes with the accumulation of sphingosine as most prominent common feature. All HNF1A erythrocyte defects could be simulated by exposure of wild type erythrocytes to sphingosine in vitro and attributed in part to sphingosine-induced suppression of the plasma-membrane Ca2 -ATPase activity. Bone marrow transplantation rescued the anemia phenotype in vivo, whereas incubation with HNF1A plasma increased the osmotic fragility of wild type erythrocytes in vitro. Our data suggest a non-cell autonomous erythrocyte defect secondary to the sphingolipid changes caused by HNF1A deficiency. Transcriptional analysis revealed four important genes involved in sphingolipid metabolism to be deregulated in HNF1A deficiency: ORMLD1, sphingosine kinase-2, neutral ceramidase and ceramide synthase-5. The considerable erythrocyte defects in murine HNF1A deficiency encourage clinical studies to explore the hematological consequences of HNF1A deficiency in human MODY3 patients.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 171107
[Lr] Last revision date:171107
[St] Status:Publisher

  5 / 885 MEDLINE  
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[PMID]: 28982343
[Au] Autor:Hagag AAE; Badraia IM; Elfarargy MS; Abd Elmageed MM; Abo-Ali EA
[Ad] Address:Pediatrics Department, Faculty of Medicine,Tanta University, Gharbia. Egypt.
[Ti] Title:Study of Glucose-6-Phosphate Dehydrogenase Deficiency: 5 Years Retrospective Egyptian study.
[So] Source:Endocr Metab Immune Disord Drug Targets;, 2017 Oct 03.
[Is] ISSN:2212-3873
[Cp] Country of publication:United Arab Emirates
[La] Language:eng
[Ab] Abstract:BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency worldwide that causes a spectrum of diseases including neonatal hyperbilirubinemia, acute and chronic hemolysis after exposure to an oxidative stress. AIM OF THE WORK: This five years retrospective study was carried out to study the demographic, clinical and laboratory data of 1000 patients with G6PD deficiency anemia registered in Hematology Unit, Pediatric Department, Tanta University Hospital. PATIENTS AND METHODS: Data were collected from patient's files, from November 2011 to November 2016, using pre-designed questionnaire to obtain complete history, clinical presentation and laboratory investigations including complete blood count, red blood cells morphology, liver and renal functions and quantitative assay of G6PD enzyme activity by spectrophotometric method. RESULTS: Males were more commonly affected than females (932 males versus 68 females). The highest prevalence of hemolytic crisis in G6PD deficiency patients was found within the age group of 1-3 years (920 patients; 92%) with mean age of first presentation of 22.8±15.54 months. Patients presented mainly with pallor (1000 patients; 100%), dark red urine (896 patients; 89.6%) and jaundice (878 patients; 87.8%) after 24-72 hours of exposure to the precipitating factors (mean: 36±17.73 hours). Diet is the most common precipitating factor of hemolysis in patients with G6PD deficiency (834 patients; 83.4% of studied cases) especially fava beans (326 patients; 32.6%) and falafel (194 patients; 19.4%) which are the most common precipitating food causing hemolysis followed by chick pea (108 patients; 10.8%), broad bean (76 patients; 7.6%), green pea (44 patients; 4.4%), pea nuts (38 patients; 3.8%), lentil (28 patients; 2.8%), and lastly black eyed peas (20 patients; 2 %). Infection was the 2nd most common cause of hemolysis (124 patients; 12.4%) including pneumonia (34 patients; 3.4%), tonsillitis (32 patients; 3.2%), typhoid fever (28 patients; 2.8%), hepatitis A (18 patients; 1.8%) and urinary tract infection (12 patients; 1.2%). Drug was the least common cause of hemolysis (42 patients; 4.2%) including diclofenac sodium (24 patients; 2.4%), ibuprofen (8 patients; 0.8%), acetylsalicylic acid (4 patients; 0.4%), co-trimoxazole (4 patients; 0.4%) and nitrofurantion (2 patients; 0.2%). There was normocytic normochromic anemia with reticulocytosis and Heinz bodies in pre-transfusion complete blood picture in all studied cases. G6PD assay show marked decrease in enzyme level at time of presentation in all cases with the commonest G6PD enzyme level of 3-4 U/gm Hb (592 patients; 59.2%). CONCLUSION AND RECOMMENDATIONS: G6PD deficiency anemia presented mainly with pallor, dark red urine and jaundice after exposure to certain diet, drugs and diseases and therefore patients with G6PD deficiency should avoid exposure to these precipitating factors of hemolysis, also we can recommend large neonatal screening programs to detect cases of G6PD deficiency before occurrence of acute hemolysis and molecular studies to detect G6PD enzyme variant in Egypt.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1710
[Cu] Class update date: 171006
[Lr] Last revision date:171006
[St] Status:Publisher
[do] DOI:10.2174/1871530317666171003160350

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[PMID]: 28969181
[Au] Autor:Virmani S; Bhat R; Rao R; Khanna R; Agarwal L
[Ad] Address:Undergraduate Student, Department of Medicine, Kasturba Medical College, Manipal University, Manipal, Karnataka, India.
[Ti] Title:A Rare Cause of Anaemia in HIV/AIDS.
[So] Source:J Clin Diagn Res;11(8):OD01-OD02, 2017 Aug.
[Is] ISSN:2249-782X
[Cp] Country of publication:India
[La] Language:eng
[Ab] Abstract:Patients living with HIV are commonly diagnosed with anaemia which can have various aetiologies. However, one of the rare causes of anaemia in such patients is Auto Immune Haemolytic Anaemia (AIHA), which is difficult to diagnose due to the absence of reticulocytosis in HIV patients. Such patients can be treated with corticosteroids which can gradually be tapered off over a period of time. The following is a case of a 52-year-old male living with HIV who was diagnosed to have AIHA and was successfully treated with steroids.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1710
[Cu] Class update date: 171006
[Lr] Last revision date:171006
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.7860/JCDR/2017/28560.10338

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[PMID]: 28870408
[Au] Autor:Moore JA; Gliga L; Nagalla S
[Ad] Address:Department of Internal Medicine, The University of Texas Southwestern Medical Center, Dallas, TX, United States. Electronic address: Joseph.Moore@phhs.org.
[Ti] Title:Thyroid storm and warm autoimmune hemolytic anemia.
[So] Source:Transfus Apher Sci;56(4):606-608, 2017 Aug.
[Is] ISSN:1473-0502
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Graves' disease is often associated with other autoimmune disorders, including rare associations with autoimmune hemolytic anemia (AIHA). We describe a unique presentation of thyroid storm and warm AIHA diagnosed concurrently in a young female with hyperthyroidism. The patient presented with nausea, vomiting, diarrhea and altered mental status. Laboratory studies revealed hemoglobin 3.9g/dL, platelets 171×10 L , haptoglobin <5mg/dL, reticulocytosis, and positive direct antiglobulin test (IgG, C3d, warm). Additional workup revealed serum thyroid stimulating hormone (TSH) <0.01µIU/mL and serum free-T4 (FT4) level 7.8ng/dL. Our patient was diagnosed with concurrent thyroid storm and warm AIHA. She was started on glucocorticoids to treat both warm AIHA and thyroid storm, as well as antithyroid medications, propranolol and folic acid. Due to profound anemia and hemodynamic instability, the patient was transfused two units of uncrossmatched packed red blood cells slowly and tolerated this well. She was discharged on methimazole as well as a prolonged prednisone taper, and achieved complete resolution of the thyrotoxicosis and anemia at one month. Hyperthyroidism can affect all three blood cell lineages of the hematopoietic system. Anemia can be seen in 10-20% of patients with thyrotoxicosis. Several autoimmune processes can lead to anemia in Graves' disease, including pernicious anemia, celiac disease, and warm AIHA. This case illustrates a rarely described presentation of a patient with Graves' disease presenting with concurrent thyroid storm and warm AIHA.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1709
[Cu] Class update date: 171003
[Lr] Last revision date:171003
[St] Status:In-Process

  8 / 885 MEDLINE  
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[PMID]: 28852037
[Au] Autor:Bancone G; Kalnoky M; Chu CS; Chowwiwat N; Kahn M; Malleret B; Wilaisrisak P; Rénia L; Domingo GJ; Nosten F
[Ad] Address:Shoklo Malaria Research Unit, Mahidol-Oxford Tropical Medicine Research Unit, Faculty of Tropical Medicine, Mahidol University, Mae Sot, Thailand. germana@tropmedres.ac.
[Ti] Title:The G6PD flow-cytometric assay is a reliable tool for diagnosis of G6PD deficiency in women and anaemic subjects.
[So] Source:Sci Rep;7(1):9822, 2017 Aug 29.
[Is] ISSN:2045-2322
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Glucose-6-phosphate dehydrogenase (G6PD) activity is essential for redox equilibrium of red blood cells (RBCs) and, when compromised, the RBCs are more susceptible to haemolysis. 8-aminoquinolines (primaquine and tafenoquine) are used for the radical curative treatment of Plasmodium vivax malaria and can cause haemolysis in G6PD deficient subjects. Haemolytic risk is dependent on treatment dose and patient G6PD status but ultimately it correlates with the number of G6PD deficient RBCs. The G6PD spectrophotometric assay reliably identifies deficient subjects but is less reliable in heterozygous females, especially when other blood conditions are present. In this work we analysed samples with a range of G6PD phenotypes and haematologic conditions from 243 healthy volunteers of Asian or African-American heritage using both the spectrophotomeric assay and the G6PD flow-cytometric assay. Overall 18.5% of subjects (29.3% of Asian females) presented with anaemia, associated with decreased RBCs volume (MCV) and reticulocytosis; the flow-cytometric assay showed good correlation with the spectrophotometric assay (Pearson's r 0.918-0.957) and was less influenced by haemoglobin concentration, number of RBCs and number of reticulocytes. This resulted in more precise quantification of the number of G6PD deficient RBCs and presumably higher predictive power of drug induced haemolytic risk.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1708
[Cu] Class update date: 170903
[Lr] Last revision date:170903
[St] Status:In-Data-Review
[do] DOI:10.1038/s41598-017-10045-2

  9 / 885 MEDLINE  
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[PMID]: 28844412
[Au] Autor:Nkya S; Mgaya J; Urio F; Makubi A; Thein SL; Menzel S; Cox SE; Newton CR; Kirkham FJ; Mmbando BP; Makani J
[Ad] Address:Sickle Cell Programme, Muhimbili University of Health and Allied Sciences, Dar-es-Salaam, Tanzania; Dar-es-Salaam University College of Education, Dar-es-Salaam, Tanzania. Electronic address: siana.nkya@duce.ac.tz.
[Ti] Title:Fetal Hemoglobin is Associated with Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzania.
[So] Source:EBioMedicine;23:146-149, 2017 Sep.
[Is] ISSN:2352-3964
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Fetal hemoglobin (HbF) and peripheral hemoglobin oxygen saturation (SpO ) both predict clinical severity in sickle cell disease (SCD), while reticulocytosis is associated with vasculopathy, but there are few data on mechanisms. HbF, SpO and routine clinical and laboratory measures were available in a Tanzanian cohort of 1175 SCD individuals aged≥5years and the association with SpO (as response variable transformed to a Poisson distribution) was assessed by negative binomial model with age and sex as covariates. Increase in HbF was associated with increased SpO (rate ratio, RR=1.19; 95% confidence intervals [CI] 1.04, 1.37 per natural log unit of HbF; p=0.0004). In univariable analysis, SpO was inversely associated with age, reticulocyte count, and log (total bilirubin) and directly with pulse, SBP, hemoglobin, and log(HbF). In multivariable regression log(HbF) (RR 1.191; 95%CI 1.04, 1.37; p=0.013), pulse (RR 1.01; 95%CI 1.00, 1.01; p=0.026), SBP (RR 1.008; 95%CI 1.00, 1.02; p=0.014), and hemoglobin (1.120; 95%CI 1.05, 1.19; p=0.001) were positively and independently associated with SpO while reticulocyte count (RR 0.985; 95%CI 0.97, 0.99; p=0.019) was independently inversely associated with SpO . In SCD, improving SpO , in part through cardiovascular compensation and associated with reduced reticulocytosis, may be a mechanism by which HbF reduces disease severity.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1708
[Cu] Class update date: 170928
[Lr] Last revision date:170928
[St] Status:In-Process

  10 / 885 MEDLINE  
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[PMID]: 28821482
[Au] Autor:Farrelly SJ; O'Connor KA
[Ad] Address:Older Persons Specialist Health Services, Mercy University Hospital, Cork, Ireland.
[Ti] Title:Hypersegmented neutrophils and oval macrocytes in the setting of B deficiency and pancytopaenia.
[So] Source:BMJ Case Rep;2017, 2017 Aug 17.
[Is] ISSN:1757-790X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Vitamin B deficiency is a recognised pathology in several populations, with a particular prevalence in an older adult population. We present two cases whereby vitamin B deficiency is the causative factor in marked pancytopaenia. Oval macrocytosis and hypersegmented neutrophils were noted on both peripheral blood samples, which are a characteristic finding in macrocytic anaemia due to B deficiency. Distinct underlying pathologies were identified in both cases; food-cobalamin malabsorption and pernicious anaemia. Parenteral vitamin B supplementation resulted in a marked reticulocytosis and rapid improvement of haematological indices in both cases. We present this series to serve as a reminder that B deficiency can present as life-threatening pancytopaenia.It has has multiple underlying pathologies,defined risk populations and has characteristic blood film findings which can guide investigations, diagnosis and treatment.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1708
[Cu] Class update date: 170819
[Lr] Last revision date:170819
[St] Status:In-Process


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